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以急性呼吸窘迫综合征为首发表现的肝血管肉瘤合并卡-梅二氏综合征 1 例报告

Acute respiratory distress syndrome as the initial symptom of hepatic angiosarcoma with Kasabach-Merritt syndrome: A case report.

机构信息

Department of Critical Care Medicine, The Second Hospital of Jilin University, Changchun, Jilin, China.

出版信息

Medicine (Baltimore). 2024 Sep 27;103(39):e39800. doi: 10.1097/MD.0000000000039800.

Abstract

RATIONALE

Hepatic angiosarcoma (HSA) has a poor prognosis. Our understanding of its clinical features, diagnosis, treatment, and prognosis remains limited. In certain cases, vascular tumors such as HSA can induce bleeding complications due to thrombocytopenia, known as Kasabach-Merritt syndrome (KMS). When KMS symptoms occur in the lungs, its clinical manifestations closely resemble those of ARDS, leading to misdiagnosis and poor outcomes. Unfortunately, this condition is extremely rare and there is a lack of relevant case reports, which further adds to the difficulty of its diagnosis and treatment.

PATIENT CONCERNS

This case report describes a patient who initially presented with symptoms of ARDS. Due to the unique nature of these symptoms, the patient underwent a complex diagnostic and treatment process before finally being diagnosed with HSA complicated by KMS through pathological examination.

DIAGNOSES

The patient was eventually diagnosed with HSA by pathology and KMS with multiorgan hemorrhage.

INTERVENTIONS

Highly misleading clinical manifestations were recorded during the diagnosis and treatment, which, to our knowledge, have not been previously reported.

OUTCOMES

The patient died from a massive pulmonary hemorrhage.

LESSONS

Dysfunction of a single organ or system may be the external manifestation of a multi-system clinical disease. Therefore, in the clinical diagnosis and treatment process, especially during early diagnosis, while it is important to focus on the primary or typical clinical symptoms, it is equally crucial not to underestimate or ignore accompanying symptoms that lack specificity. When diagnosis and treatment reach an impasse, these "atypical" symptoms often prove to be key in solving the puzzle.

摘要

背景

肝血管肉瘤(HSA)预后较差。我们对其临床特征、诊断、治疗和预后的了解仍然有限。在某些情况下,HSA 等血管肿瘤由于血小板减少会引起出血并发症,称为卡-梅(Kasabach-Merritt)综合征(KMS)。当 KMS 症状发生在肺部时,其临床表现与 ARDS 非常相似,导致误诊和不良结局。不幸的是,这种情况非常罕见,而且缺乏相关的病例报告,这进一步增加了其诊断和治疗的难度。

患者关注

本病例报告描述了一位最初表现为 ARDS 症状的患者。由于这些症状的独特性质,患者在最终通过病理检查诊断为 HSA 合并 KMS 之前经历了复杂的诊断和治疗过程。

诊断

患者最终通过病理学诊断为 HSA 和 KMS 合并多器官出血。

干预措施

在诊断和治疗过程中记录了高度误导的临床表现,据我们所知,这些临床表现以前没有报道过。

结果

患者死于大量肺出血。

教训

单个器官或系统的功能障碍可能是多系统临床疾病的外在表现。因此,在临床诊断和治疗过程中,特别是在早期诊断时,虽然关注主要或典型的临床症状很重要,但同样重要的是不要低估或忽视伴随的缺乏特异性的症状。当诊断和治疗陷入僵局时,这些“非典型”症状往往是解决问题的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efab/11441907/fdd57460d781/medi-103-e39800-g001.jpg

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